Families members with or at risk for Nevoid Basal Cell Carcinoma Syndrome (NBCC) are being evaluated in the Audiology Clinic to assess auditory system function in this population. Hearing loss has been mentioned in the literature, but has not been seriously investigated. As of 1 May 1985, approximately 25 patients (affected and at-risk first degree relatives) have been seen for studies of puretone and speech thresholds, suprathreshold recognition for speech and middle ear analysis including acoustic reflex measurements. We are just beginning to include the study of auditory evoked responses in this population. Very early results are being tabulated for presentation at the Birth Defects & Genetics Meeting (Boston; May, 1985). As this study is just in its early phases, we anticipate considerably more data to evaluate which should provide better insight into the auditory system anomalies in NBCC.